Podcast
Questions and Answers
Disease of the kidney are divided into four categories depending on which component of the kidney is primarily affected. Which of the following is NOT one of those categories?
Disease of the kidney are divided into four categories depending on which component of the kidney is primarily affected. Which of the following is NOT one of those categories?
Glomerular disease is often immunologically mediated.
Glomerular disease is often immunologically mediated.
True
Tubular and interstitial disorders are more likely to be caused by infectious agents than glomerular disease.
Tubular and interstitial disorders are more likely to be caused by infectious agents than glomerular disease.
True
Nephrotic syndrome is characterized by which of the following?
Nephrotic syndrome is characterized by which of the following?
Signup and view all the answers
Which of the following is NOT a symptom associated with Nephritic syndrome?
Which of the following is NOT a symptom associated with Nephritic syndrome?
Signup and view all the answers
Berger's disease, also known as IgA nephropathy, is the most common cause of primary glomerulonephritis.
Berger's disease, also known as IgA nephropathy, is the most common cause of primary glomerulonephritis.
Signup and view all the answers
The early manifestations of each group of kidney diseases tend to be ______.
The early manifestations of each group of kidney diseases tend to be ______.
Signup and view all the answers
What is the name for the group of diseases that involve the formation of cysts in the kidneys?
What is the name for the group of diseases that involve the formation of cysts in the kidneys?
Signup and view all the answers
Cystic kidney diseases are a heterogeneous group and can be hereditary, developmental, or acquired.
Cystic kidney diseases are a heterogeneous group and can be hereditary, developmental, or acquired.
Signup and view all the answers
Why are cystic kidney diseases considered important?
Why are cystic kidney diseases considered important?
Signup and view all the answers
Autosomal dominant polycystic kidney disease is characterized by:
Autosomal dominant polycystic kidney disease is characterized by:
Signup and view all the answers
The mode of inheritance for autosomal dominant polycystic kidney disease is autosomal recessive.
The mode of inheritance for autosomal dominant polycystic kidney disease is autosomal recessive.
Signup and view all the answers
What genes are associated with autosomal dominant polycystic kidney disease?
What genes are associated with autosomal dominant polycystic kidney disease?
Signup and view all the answers
Mutations in PKD1 are responsible for more severe cases of autosomal dominant polycystic kidney disease.
Mutations in PKD1 are responsible for more severe cases of autosomal dominant polycystic kidney disease.
Signup and view all the answers
What type of cells does Polycystin-1 localize to?
What type of cells does Polycystin-1 localize to?
Signup and view all the answers
Autosomal recessive polycystic kidney disease is more common than autosomal dominant polycystic kidney disease.
Autosomal recessive polycystic kidney disease is more common than autosomal dominant polycystic kidney disease.
Signup and view all the answers
Autosomal recessive polycystic kidney disease is subcategorized based on:
Autosomal recessive polycystic kidney disease is subcategorized based on:
Signup and view all the answers
The gene associated with autosomal recessive polycystic kidney disease is called PKHD1.
The gene associated with autosomal recessive polycystic kidney disease is called PKHD1.
Signup and view all the answers
Which of the following is NOT a common extrarenal manifestation of autosomal dominant polycystic kidney disease?
Which of the following is NOT a common extrarenal manifestation of autosomal dominant polycystic kidney disease?
Signup and view all the answers
Glomerular diseases are among the most common causes of Chronic kidney failure in humans.
Glomerular diseases are among the most common causes of Chronic kidney failure in humans.
Signup and view all the answers
The pathogenesis of glomerular diseases is primarily due to immune mechanisms.
The pathogenesis of glomerular diseases is primarily due to immune mechanisms.
Signup and view all the answers
What are the two main histologic characteristics of glomerular diseases?
What are the two main histologic characteristics of glomerular diseases?
Signup and view all the answers
Which of the following is a secondary (systemic) disease associated with glomerular diseases?
Which of the following is a secondary (systemic) disease associated with glomerular diseases?
Signup and view all the answers
Minimal Change Disease (lipoid nephrosis) is a rare glomerular disease.
Minimal Change Disease (lipoid nephrosis) is a rare glomerular disease.
Signup and view all the answers
Minimal Change Disease is primarily associated with adults, specifically those between 30-50 years old.
Minimal Change Disease is primarily associated with adults, specifically those between 30-50 years old.
Signup and view all the answers
Minimal Change Disease is characterized by diffuse thickening of the glomerular basement membrane.
Minimal Change Disease is characterized by diffuse thickening of the glomerular basement membrane.
Signup and view all the answers
Minimal Change Disease often leads to a rapid decline in renal function.
Minimal Change Disease often leads to a rapid decline in renal function.
Signup and view all the answers
Treatment with corticosteroids is often effective in managing Minimal Change Disease.
Treatment with corticosteroids is often effective in managing Minimal Change Disease.
Signup and view all the answers
Membranous glomerulonephropathy is a common cause of nephrotic syndrome in adults.
Membranous glomerulonephropathy is a common cause of nephrotic syndrome in adults.
Signup and view all the answers
Membranous glomerulonephropathy is usually caused by an immune response to a specific infectious agent.
Membranous glomerulonephropathy is usually caused by an immune response to a specific infectious agent.
Signup and view all the answers
The formation of the membrane attack complex is linked to the action of C5b-C9.
The formation of the membrane attack complex is linked to the action of C5b-C9.
Signup and view all the answers
Membranous glomerulonephropathy is more commonly associated with secondary forms than primary forms.
Membranous glomerulonephropathy is more commonly associated with secondary forms than primary forms.
Signup and view all the answers
Which of the following is NOT a common secondary form of membranous glomerulonephropathy?
Which of the following is NOT a common secondary form of membranous glomerulonephropathy?
Signup and view all the answers
Membranous glomerulonephropathy is characterized by a diffuse thickening of the glomerular capillary wall.
Membranous glomerulonephropathy is characterized by a diffuse thickening of the glomerular capillary wall.
Signup and view all the answers
Electron microscopy reveals granular deposits in the subepithelial side of the basement membrane in membranous glomerulonephropathy.
Electron microscopy reveals granular deposits in the subepithelial side of the basement membrane in membranous glomerulonephropathy.
Signup and view all the answers
Membranous glomerulonephropathy is associated with poor prognosis and a high risk of rapidly progressing to kidney failure.
Membranous glomerulonephropathy is associated with poor prognosis and a high risk of rapidly progressing to kidney failure.
Signup and view all the answers
Focal segmental glomerulosclerosis is characterized by scarring of the glomeruli.
Focal segmental glomerulosclerosis is characterized by scarring of the glomeruli.
Signup and view all the answers
Focal segmental glomerulosclerosis is typically considered a primary glomerular disease.
Focal segmental glomerulosclerosis is typically considered a primary glomerular disease.
Signup and view all the answers
Focal segmental glomerulosclerosis often presents with a high incidence of hematuria and hypertension.
Focal segmental glomerulosclerosis often presents with a high incidence of hematuria and hypertension.
Signup and view all the answers
Patients with Focal segmental glomerulosclerosis often respond well to corticosteroid therapy.
Patients with Focal segmental glomerulosclerosis often respond well to corticosteroid therapy.
Signup and view all the answers
Focal segmental glomerulosclerosis is characterized by a diffuse thickening of the glomerular basement membrane.
Focal segmental glomerulosclerosis is characterized by a diffuse thickening of the glomerular basement membrane.
Signup and view all the answers
Which of the following is NOT a common setting in which Focal segmental glomerulosclerosis can occur?
Which of the following is NOT a common setting in which Focal segmental glomerulosclerosis can occur?
Signup and view all the answers
Focal segmental glomerulosclerosis often progresses to global glomerular sclerosis and kidney failure.
Focal segmental glomerulosclerosis often progresses to global glomerular sclerosis and kidney failure.
Signup and view all the answers
Poststreptococcal glomerulonephritis is characterized by a rapid onset, typically occurring within 1-4 weeks after a streptococcal infection.
Poststreptococcal glomerulonephritis is characterized by a rapid onset, typically occurring within 1-4 weeks after a streptococcal infection.
Signup and view all the answers
Poststreptococcal glomerulonephritis primarily affects adults.
Poststreptococcal glomerulonephritis primarily affects adults.
Signup and view all the answers
Only Type A streptococci are nephrogenic.
Only Type A streptococci are nephrogenic.
Signup and view all the answers
Poststreptococcal glomerulonephritis typically presents with a classic symptom triad of: fever, malaise, and oliguria.
Poststreptococcal glomerulonephritis typically presents with a classic symptom triad of: fever, malaise, and oliguria.
Signup and view all the answers
Poststreptococcal glomerulonephritis often exhibits hyperlipidemia.
Poststreptococcal glomerulonephritis often exhibits hyperlipidemia.
Signup and view all the answers
The pathogenesis of Poststreptococcal glomerulonephritis is primarily due to immune complex formation against streptococcal antigens.
The pathogenesis of Poststreptococcal glomerulonephritis is primarily due to immune complex formation against streptococcal antigens.
Signup and view all the answers
Poststreptococcal glomerulonephritis is characterized by a uniform increase in cellularity within the glomeruli.
Poststreptococcal glomerulonephritis is characterized by a uniform increase in cellularity within the glomeruli.
Signup and view all the answers
Electron microscopy often reveals subepithelial humps in Poststreptococcal glomerulonephritis, indicating immune complex deposits above the basement membrane.
Electron microscopy often reveals subepithelial humps in Poststreptococcal glomerulonephritis, indicating immune complex deposits above the basement membrane.
Signup and view all the answers
The prognosis for Poststreptococcal glomerulonephritis is excellent, with most cases leading to complete recovery.
The prognosis for Poststreptococcal glomerulonephritis is excellent, with most cases leading to complete recovery.
Signup and view all the answers
IgA nephropathy (Berger's disease) is a rare form of glomerulonephritis.
IgA nephropathy (Berger's disease) is a rare form of glomerulonephritis.
Signup and view all the answers
IgA nephropathy is primarily associated with the deposition of IgA within the mesangium.
IgA nephropathy is primarily associated with the deposition of IgA within the mesangium.
Signup and view all the answers
IgA nephropathy often presents with recurrent hematuria.
IgA nephropathy often presents with recurrent hematuria.
Signup and view all the answers
IgA nephropathy is often associated with a recent upper respiratory tract infection.
IgA nephropathy is often associated with a recent upper respiratory tract infection.
Signup and view all the answers
The pathogenesis of IgA nephropathy is linked to a deficiency in IgA.
The pathogenesis of IgA nephropathy is linked to a deficiency in IgA.
Signup and view all the answers
IgA nephropathy is characterized by a variable degree of mesangial proliferation.
IgA nephropathy is characterized by a variable degree of mesangial proliferation.
Signup and view all the answers
Electron microscopy can help identify the presence of electron-dense deposits within the mesangium in IgA nephropathy.
Electron microscopy can help identify the presence of electron-dense deposits within the mesangium in IgA nephropathy.
Signup and view all the answers
Study Notes
Renal System Pathology 1
- The renal system is divided into four categories based on the primarily affected component: glomerular, tubular, interstitial, and vascular.
- Early manifestations of each group of kidney diseases are distinctive.
- Glomerular diseases are often immunologically mediated, while tubular and interstitial disorders are more likely caused by toxic or infectious agents.
- Chronic renal disease eventually damages all four components, leading to chronic renal failure (end-stage kidney disease).
Nephrotic Syndrome
- Characterized by proteinuria (3.5 g/day or more in adults), hypoalbuminemia (less than 3 g/dL), generalized edema, and hyperlipidemia/lipiduria.
- Symptoms include edema (starts in the face), pale skin, tachycardia, vomiting
Nephritic Syndrome
- Characterized by hematuria (blood in the urine) and red blood cell casts, proteinuria (usually in the sub-nephrotic range), azotemia (abnormally high nitrogen levels), and hypertension.
- Symptoms include hematuria, blurred vision, oliguria (low output of urine), and hypertension
Cystic Diseases of the Kidney
- These are heterogeneous disorders including hereditary, developmental, and acquired conditions.
- They represent significant diagnostic challenges for clinicians, radiologists, and pathologists and can be major causes of chronic renal failure
- They can be confused with malignant tumors.
- Includes: cystic renal dysplasia, polycystic kidney disease (autosomal dominant and autosomal recessive), medullary cystic disease (medullary sponge kidney and nephronophthisis), acquired (dialysis-associated) cystic disease, and renal cysts in hereditary malformation syndromes (e.g., tuberous sclerosis). Includes parasitic cysts (e.g., hydatid cyst)
Autosomal Dominant Polycystic Kidney Disease (ADPKD)
- Characterized by multiple cysts in both kidneys.
- The cysts enlarge, destroying the renal parenchyma leading to renal failure.
- Affects about 1 in 400 to 1000 live births; accounts for 5-10% of chronic renal failure requiring transplantation or dialysis.
- The mode of inheritance is autosomal dominant, high penetrance
- The disease is universally bilateral.
Autosomal Recessive Polycystic Kidney Disease (ARPKD)
-
A rare developmental anomaly.
-
Subcategorized based on presentation time (perinatal, neonatal, infantile, juvenile) and liver involvement.
-
The first two subtypes (perinatal and neonatal) are the most common.
-
Inheritance is autosomal recessive with a gene (PKHD1) mapping to chromosome 6p21-23.
-
Typically, shows enlarged kidneys with numerous small cysts in the cortex and medulla.
Glomerular Diseases
- Immune mechanisms (antibody-based and cellular) underlie most primary and secondary glomerular diseases.
- Histological changes of glomerular diseases are classified as diffuse, focal, global, segmental, and mesangial.
- Categorization considers the proportion of affected glomeruli.
Immune-mediated Mechanisms
- In situ immune complex deposition
- Fixed intrinsic antigens
- Planted antigens (external pathogens or drugs)
- Endogenous antigens (DNA, proteins)
Non-immune Mechanisms:
- Focal segmental glomerulosclerosis (FSGS)
- Tubulointerstitial fibrosis
Minimal Change Disease
- The most frequent cause of nephrotic syndrome in children.
- Characterized by normal glomeruli under light microscopy, and diffuse effacement of foot processes under electron microscopy.
- It's benign and most children respond to steroid therapy.
Membranous Glomerulonephropathy
- Common cause of nephrotic syndrome in adults (ages 30–50).
- Typically caused by chronic immune complex-mediated disease due to antibodies against renal GBM autoantigens.
- Idiopathic in 85% of cases
- It is characterized by diffuse thickening of the glomerular basement membrane, with subepithelial deposits (immune deposits) appearing as spikes, and loss of foot processes.
IgA Nephropathy (Berger's Disease)
- Frequent cause of recurrent gross or microscopic hematuria.
- Typically characterized by variable mesangial proliferation with IgA in mesangium.
Acute Postinfectious Post-streptococcal GN
- Develops 1-4 weeks after a streptococcal infection (pharynx or skin).
- Mostly affects children aged 6–10 years, but adults can get it.
- Certain strains of group A, beta-hemolytic streptococci are nephrogenic.
- Characterized by acute inflammation and proliferation of glomerular cells.
Studying That Suits You
Use AI to generate personalized quizzes and flashcards to suit your learning preferences.
Related Documents
Description
Test your knowledge on the renal system and its pathologies in this comprehensive quiz. Explore the differences between nephrotic and nephritic syndromes, as well as the categories of kidney diseases. Assess your understanding of the early manifestations and the underlying causes of these conditions.